神经精神性狼疮伴黑棘皮病的不典型表现。

Atypical presentation of neuropsychiatric lupus with acanthosis nigricans.

机构信息

Department of Dermatology, Necker-Enfants Malades Hospital, Assistance Publique-Hopitaux de Paris, Paris Descartes University, Paris, France.

出版信息

Pediatr Neurol. 2012 Oct;47(4):291-4. doi: 10.1016/j.pediatrneurol.2012.06.005.

DOI:10.1016/j.pediatrneurol.2012.06.005

PMID:22964444

Abstract

Cutaneous manifestations are commonly observed in pediatric patients with systemic lupus erythematosus. Acanthosis nigricans, however, is rarely reported, and even less often in association with neuropsychiatric manifestations of lupus erythematosus. We describe a 9-year-old boy with acute behavioral and cognitive deterioration, combined with cutaneous, diffuse hyperpigmented and hyperkeratotic plaques. Cerebral magnetic resonance imaging revealed cortical atrophy and white matter abnormalities involving the fornix, corpus callosum, and parieto-occipital periventricular regions. The presence of progressive cognitive and behavioral deterioration, combined with abnormal white matter signals on magnetic resonance imaging, led us to suspect X-linked adrenoleukodystrophy. The subsequent development of systemic signs, together with positive serologic tests, confirmed the diagnosis of neuropsychiatric lupus with acanthosis nigricans. We review the literature on acanthosis nigricans in systemic lupus erythematosus and the value of magnetic resonance imaging in evaluating patients with neuropsychiatric systemic lupus.

摘要

皮肤表现通常在患有系统性红斑狼疮的儿科患者中观察到。然而，黑棘皮病很少被报道，并且在与系统性红斑狼疮的神经精神表现相关时更为少见。我们描述了一例 9 岁男孩，他出现急性行为和认知功能恶化，伴有皮肤弥漫性色素沉着和角化过度斑块。大脑磁共振成像显示皮质萎缩和白质异常，累及穹窿、胼胝体和顶枕叶脑室周围区域。进行性认知和行为恶化，加上磁共振成像上的异常白质信号，使我们怀疑 X 连锁肾上腺脑白质营养不良。随后出现全身征象，加上阳性血清学检查，证实了伴有黑棘皮病的神经精神性狼疮的诊断。我们回顾了系统性红斑狼疮中黑棘皮病的文献以及磁共振成像在评估神经精神性系统性红斑狼疮患者中的价值。